Operative management of pulmonary abscess due to spontaneous perforation of diffuse intramural esophageal pseudodiverticulosis

Abstract

Diffuse intramural esophageal pseudodiverticulosis is a rare, benign esophageal condition of unclear cause, pathologically manifested by pseudodiverticula along a portion of the esophagus. It most commonly presents as dysphagia and is usually amenable to endoscopic dilation. It is most reliably diagnosed endoscopically or with a fluoroscopic swallow study. Surgical intervention is rarely indicated but can create a need for an extensive resection and reconstruction, as in the case presented here.

Diffuse intramural esophageal pseudodiverticulosis (DIEP) is an uncommon condition of the esophagus first described in 1960 (1). The diverticula were initially thought to consist of all esophageal layers, but more advanced pathologic and histologic examination revealed pseudodiverticula consisting only of mucosa. The hallmark of the disorder is the presence of dilated excretory ducts of the submucosal glands leading to the radiographic appearance of tortuosity with multiple small outpouchings. The most common symptom is dysphagia due to a benign stricture, usually amenable to endoscopic dilation. The condition is associated with diabetes, cirrhosis, gastroesophageal reflux, and candidosis. Any need for operative intervention for this uncommon entity is exceedingly rare.

We present a case of a 56-year-old woman presenting with spontaneous pulmonary abscesses who was found to have DIEP by radiographic and endoscopic evaluation. The patient underwent an esophageal resection with placement of feeding tubes and formation of a cervical esophagostomy and drainage of the abscess, followed by nutritional optimization at a rehabilitation center. She then was readmitted electively to the hospital to undergo a transhiatal gastric pull-up procedure via a laparotomy with a neck exploration and anastomosis to her remnant cervical esophagus. This procedure usually leaves the neoesophagus lying in the former location of the diseased esophagus, but our patient's history of pulmonary abscesses had led to chronic inflammatory changes in that location. To facilitate reconstruction, the stomach (neoesophagus) was placed in a retrosternal position anterior to the heart and mediastinum. This restored intestinal continuity without the difficult dissection and inherent dangers of operating on tissue scarred by infection and inflammation. We describe the patient's presentation, work-up, and operative management and review this rare esophageal condition.

CASE PRESENTATION

A 56-year-old African American woman presented with a 110-pound weight loss, decreased appetite, weakness, and nausea. A computed tomography (CT) scan of the abdomen revealed a thickened gastric antrum, and an esophagogastroduodenoscopy (EGD) demonstrated severe mucosal abnormalities of the entire esophagus, presumed at that time to be secondary to a Candida infection. A fluoroscopic swallow study demonstrated a pathologically dilated and irregular esophagus (Figure 1).

Figure 1.

Fluoroscopic swallow study showing a pathologically dilated and irregular esophagus.

The patient also had a small incidental mass in her distal esophagus. Biopsies revealed a benign papilloma. Gradually her condition stabilized, but then she was unfortunately lost to follow up for several months. When she again presented to the emergency department, she complained of dysphagia, productive cough, dark sputum, right-sided chest pain, and shortness of breath. On physical examination, she was cachectic and appeared older than her stated age. Rhonci and crackles were appreciable in her right lung base, as well as decreased breath sounds overall. Her white blood cell count was 30,000/mL with a bandemia of 10%. At admission her blood pressure was 103/66 mm Hg, her heart rate was 95 beats per minute, and her oxygen saturation was 98%. A CT scan demonstrated a right lower lobe pulmonary abscess (Figure 2). Another EGD revealed four areas suspicious for perforations, and an esophagram demonstrated findings suspicious for a communicating tract from the esophagus to the right chest (Figure 3).

Figure 2.

CT scan demonstrating a right lower lobe pulmonary abscess.

Figure 3.

Esophagogastroduodenoscopy revealing four areas suspicious for perforations.

At this time she underwent a right thoracotomy and exploratory laparotomy to resect the diseased esophagus and proximal stomach. A decompressive gastrostomy tube and feeding jejunostomy tube were placed. The proximal esophagus was drained with a Silastic tube, which was later revised into a cervical esophagostomy. At the time of her initial operation, her prealbumin was 5.1 mg/dL. After her initial acute postoperative phase, she was discharged to a nursing home for further rehabilitation to prepare her for a definitive repair.

She returned to the hospital approximately 6 weeks later. At that time, her white blood cell count was normal, her prealbumin was 23 mg/dL, and she had become accustomed to her tube feeding regimen and reported an overall improvement in her health. She underwent an exploratory laparotomy with mobilization of her stomach with preservation of the right gastroepiploic artery as well as a mobilization of the proximal end of the esophagostomy in her left neck. The abundant natural collateral vascular system of the stomach allows sacrifice of both right and left gastric arteries as well as the left gastroepiploic artery, allowing the stomach to function on the pedicle of the right gastroepiploic artery alone. As this permits a large degree of freedom, the proximal stomach can reach surprising heights in the neck and support the metabolic demands of a healing anastomosis. This portion of the operation was performed via a midline laparotomy, which allowed dissection of surgical planes up to and past the diaphragmatic hiatus from below. The cervical esophagostomy, or “spit fistula,” was separated from the skin and freed up from all the scar tissue underneath, taking care to avoid the great vessels and important nerves in the neck.

After the esophageal remnant had been sufficiently mobilized, a retrosternal tunnel was developed with the use of a 90-degree mediastinoscope and was extended until it connected with the dissection extending superiorly from the hiatus. The CT scans obtained preoperatively suggested there would be massive inflammatory changes and scarring in the native esophageal bed (Figure 4). The use of a retrosternal tunnel avoided the need to dissect through pathologically altered tissues.

Figure 4.

Preoperative CT scan suggesting massive inflammatory changes and scarring in the native esophageal tract surrounded by an inflamed lung (arrow). The X shows the retrosternal position of the neoesophagus.

The stomach was then passed through this tunnel, and a single-layer hand-sewn anastomosis was created with the esophageal stump after careful placement of a nasogastric tube under direct vision. A pyloromyotomy was performed, a new jejunostomy tube was placed, and the abdomen was closed. The cervical incision was closed over a Penrose drain, as the wound had been exposed to the interior of the enteric tract. The patient was slowly restarted on her tube feeds on postoperative day 3. She received a brief stint of parenteral nutrition in the meantime. On postoperative day 6, when she was afebrile with a normal white count and an esophagram showed no evidence of a leak, her nasogastric tube was removed (Figure 5). She began tolerating a full liquid diet and mobilized well and was soon discharged home.

Figure 5.

Final fluoroscopic imaging showing no evidence of a leak.

DISCUSSION

DIEP is a rare condition, with approximately 100 cases presented in the literature. When it was first described by Mendl in 1960 (1), the diverticula were wrongly thought to be “true,” i.e., full-thickness diverticula. Further advancement in the field of pathology and histology revealed pathologically dilated submucosal glands forming pseudodiverticula into the muscular layer of the esophagus. Multiple theories exist as to the pathogenesis of these pseudodiverticula, including occlusion of the lumen of these ducts by desquamated cells of the mucosal glands followed by engorgement versus metaplasia of the epithelium. Other theories include underlying motility disorders or Candida infection, though these are thought to be less likely.

The condition occurs in all age groups, predominantly in the sixth and seventh decades, affecting slightly more men than women (2). The distribution of disease is segmental in about 60% and diffuse in about 40% (2). The most common presentation is progressive or intermittent dysphagia, often secondary to a benign stricture (3). The degree of dysphagia doesn't necessarily correlate with the size or extent of the diverticula (3). The dysphagia may be new or may be a chronic symptom, and it may be absent in 10% of patients (4). The diagnosis may be made radiographically or endoscopically. On barium swallow, a tortuous esophagus with multiple outpouchings will be seen (3, 5–7). The esophagus may appear to be wider than normal. Endoscopically, multiple small mucosal defects may be seen, though they are reportedly missed in 80% of endoscopies (8). In one study, the tracks had an average length of 1.2 cm (length range, 0.3–7 cm) and an average width of 1.6 mm (width range, 1–4 mm) (9). Sometimes a yellowish exudate is expressed from the openings (6). The findings may be similar to Candida esophagitis, leading to an initial misdiagnosis, as in our patient. Candida is often present in the specimen, but its role as a causative agent is doubtful, and it is more likely to be a saprophyte (10). Biopsies may be taken and will show either normal mucosa or a degree of esophagitis, often either associated with Candida or a bacterium (6, 11, 12).

Most strictures will respond to balloon dilation. One case reported a patient with documented high-amplitude contractions who benefited from an esophagomyotomy (10). Another with a similar situation benefited from a calcium-channel blocker (13). There are few reported cases of surgical intervention for DIEP; two such patients received esophagectomies, one for intractable symptoms of dysphagia and vomiting for months, and one for a mediastinal abscess and communicating fistula. Both experienced satisfactory recoveries and follow up (14, 15). Another surgically managed case of DIEP involved an esophageal perforation secondary to vomiting. The perforation proximal to a distal stricture required esophagectomy, with the diagnosis discovered after the initial operation (16).